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May 27, 2010

Neuro Pathos

[This was written, today, to be sent as an email to some friends of mine. I’ve adapted it slightly for publication on this blog. —SB]

I am aware that there are those of you who have now heard something about my little health mystery, but haven’t heard anything directly from me. I’ve talked to some of you in a rather haphazard fashion (if you were unlucky enough to stand near me on a day when I was in a mood to talk about it—and when don’t I want to talk?—you got an earful). It’s been pretty random. Some of my oldest friends haven’t heard anything from me; others who probably would have trouble picking me out of a lineup have heard it all. I probably should have kept quiet in the first place, but I didn’t and I’m losing track of what I’ve said to whom, so I might as well just put it out there.

Here’s the story, and I’ll try to be brief. [Note: I wasn’t successful.]

I’m seeing a neurologist for diagnosis of a “motor neuropathy.” Saw a GP in February (routine physical) and reported to him that I had noticed cramping in my left hand going back to spring of 2009, increasing weakness, and some trembling in the hand. He took one look at me and saw muscular atrophy in my left hand and arm. Referred me for an EMG and said this was probably a simple matter of a pinched nerve in my neck.

Well, it’s almost four months later now, and it hasn’t turned out to be a simple matter at all. I’ve now had two (abnormal) EMGs, two MRIs covering everything from my waist up, numerous blood tests, and three exams (two w/ neuro, and one with a cervical/spine surgeon). I’m also experiencing fasciculations (twitches) all over my upper body (most prominently in my left arm).

Lots of things have been considered and—most of them—rejected. The good news is that I don’t have tumors, AIDS, syphilis, any signs of cancer, or a host of other bad things. On the whole, I’m in excellent health!

At my most recent appointment, my neuro told me and Joann that “unfortunately, I think ALS is in the picture.” She said that multifocal motor neuropathy (MMN) and monomelic amyotophy are the only other possibilities she sees. Both of these are less common than ALS (in fact, monomelic amyotrophy is usually a disease of young males in parts Asia, and it rarely if ever presents any fasciculations beyond the atrophied limb). After telling us there were only these three possibilities (one of which is a stretch), she immediately discounted MMN as unlikely because “you do not have motor conduction block” (which is very often a feature). She clearly believes that I will eventually be diagnosed with ALS. She told us (twice), that she can’t diagnose me with it because I don’t yet meet all of the diagnostic criteria. The second time she said it, she looked at us and said “you understand, now, that I cannot give you that diagnosis today” as if to imply “but that’s what you have.”

She may be right. For a variety of reasons, though, I’m not convinced, and I think that MMN is still very much in the picture. I think she is a good doctor, well-meaning, and compassionate. But she is not a specialist in neuro-muscular diseases, and I know that there are good reasons for uncertainty. I don’t know that either of the nerve conduction studies done (as part of the EMG) was comprehensive enough to rule out motor conduction block. And I know that motor conduction block is not always found in MMN.

At any rate, if I am in the early stages of ALS, I have an uncommon form of it. In fact, I would be a candidate for progressive muscular atrophy (PMA), which is sometimes called “lower motor neuron predominant ALS.” (The medical community is not in agreement about whether ALS, PMA and two other variants are distinct conditions or not. In Britain, for example, they are all called “motor neuron disease” (MND) and PMA is referred to as one of the “slowly progressing forms of MND.”)

So far at least (a year after the onset of symptoms), I show no definite signs of upper motor neuron involvement. This is unusual, and it precludes a diagnosis of “classic ALS.” PMA’s prognosis is somewhat better, depending upon what you read (it’s not great).

At this point, my neuro is trying to get me a referral to a specialist at the U of M. My first choice, and the guy she mentioned initially, is “not taking new patients at this time.” That’s too bad, because he is a very highly regarded authority on motor neuropathies (in fact, he co-authored the paper in 1988 that first described MMN). But I should get a specialist in the U of M’s neurology department, and that’s a good thing.

I hope to get a definite diagnosis soon, but that may not be possible. In fact, the longer I go without a diagnosis, the better the news. MND (ALS/PMA etc) generally progresses quickly. This progression (spreading from one area of the body to others) is an important part of the diagnosis. It’s already been about a year since the onset of symptoms, and the longer I go without much progression, the better my chances to be above ground five years from now. So I’m hoping to keep drawing blanks, so to speak (to abuse a Scrabble analogy).

Wish me luck!

[If you are curious about PMA, Wikipedia is as good as anyplace to start (en.wikipedia.org/wiki/Progressive_muscular_atrophy). The “4% of ALS/MND” statement is probably on the low side. There is no MMN page at Wikipedia (it gets a shout out on the ALS page). The John’s Hopkins page (www.neuro.jhmi.edu) is OK.]

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May 27, 2010

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